A Review and Update on CholangiocarcinomaOlnes M.J.a · Erlich R.b
aDepartment of Internal Medicine, Johns Hopkins Bayview Medical Center and Johns Hopkins School of Medicine, and bDivision of Hematology-Oncology, Department of Medicine, Alvin and Lois Lapidus Cancer Institute, Sinai Hospital of Baltimore, Baltimore, Md., USA
Cholangiocarcinoma is a malignant neoplasm arising from the biliary epithelium that was first described by Durand-Fardel in 1840. Today, it continues to defy diagnosis and treatment. It is difficult to diagnose in part because of its relative rarity, and because it is clinically silent until it becomes advanced disease with obstructive symptoms. The worldwide incidence of cholangiocarcinoma has risen over the past three decades. There is marked geographic variability in the prevalence of this disease, due in large part to regional environmental risk factors. Surgical resection remains the only curative treatment, and high priorities are improving diagnostic methods, and clinical staging for resection once the disease is suspected. A recent trend towards aggressive surgical management has improved outcomes. Chemotherapy, palliative stenting, and radiation are reserved for patients who are not resectable, those with recurrence after surgery, and those who decline surgical intervention. Recent trials using combination systemic chemotherapy and neoadjuvant chemoradiation are promising, but require further study. Over the past five years, several important studies have yielded new insights into the molecular mechanisms of cholangiocarcinoma tumorigenesis. In this review we discuss epidemiology, etiologic factors, molecular pathogenesis, diagnosis, staging, and treatment of cholangiocarcinoma. Particular focus is on recent studies into the cellular and molecular pathogenesis of the disease, recent chemotherapy trials, and newer methods of staging and screening for this devastating malignancy.
© 2004 S. Karger AG, Basel
Received: January 30, 2003
Accepted after revision: June 30, 2003
Number of Print Pages : 13
Number of Figures : 1, Number of Tables : 6, Number of References : 128
Oncology (International Journal of Cancer Research and Treatment)
Founded 1948 as ‘Oncologia’ by H.R. Schinz; Continued by V. Richards (1967–1975), H. Wrba (1976–1992), P.P. Carbone (1993–2002)
Vol. 66, No. 3, Year 2004 (Cover Date: Released June 2004)
Journal Editor: D.L. Trump, Buffalo, N.Y.
ISSN: 0030–2414 (print), 1423–0232 (Online)
For additional information: http://www.karger.com/onc